BMC NephrologyPub Date : 2025-05-19DOI: 10.1186/s12882-025-04165-5
Yuehong Wang, Zhimin Wu, Liuqi Huang, Dan Suo, Min Zhang, Meifen Dai, Tianhui You, Jing Zheng
{"title":"A nomogram for predicting the risk of peritoneal dialysis-associated peritonitis in patients with end-stage renal disease undergoing peritoneal dialysis: model development and validation study.","authors":"Yuehong Wang, Zhimin Wu, Liuqi Huang, Dan Suo, Min Zhang, Meifen Dai, Tianhui You, Jing Zheng","doi":"10.1186/s12882-025-04165-5","DOIUrl":"10.1186/s12882-025-04165-5","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to develop and validate a nomogram to predict the risk of peritoneal dialysis-associated peritonitis (PDAP) in patients undergoing peritopreneal dialysis.</p><p><strong>Methods: </strong>A retrospective analysis was conducted on clinical data from 376 patients at Nanhai District People's Hospital in Foshan City, Guangdong Province, between December 2017 and December 2024. The dataset was randomly divided into a training set (n = 244) and a validation set (n = 132). Risk factors for PDAP were identified using Least Absolute Shrinkage and Selection Operator (LASSO) regression and logistic regression, and a predictive nomogram was developed and validated using R4.1.3. The model's performance was evaluated through receiver operating characteristic (ROC) curves, the Hosmer-Lemeshow goodness-of-fit test, decision curve analysis (DCA), and clinical impact curves (CICs).</p><p><strong>Results: </strong>Eight potential predictors were selected by LASSO regression analysis. Multivariate logistic regression analysis confirmed that age, dialysis duration, albumin, hemoglobin, β<sub>2</sub>-microglobulin, Potassium and lymphocyte count were independent risk factors for PDAP occurrence (P = 0.001). The nomogram's area under the curve (AUC) was 0.929 (95% CI: 0.896-0.962) in the training set and 0.905 (95% CI: 0.855-0.955) in the validation set. The Hosmer-Lemeshow goodness-of-fit test indicated a good model fit (training set χ<sup>2</sup> = 13.181, P = 0.106; validation set χ<sup>2</sup> = 8.264, P = 0.408). Both DCA and CIC revealed that the nomogram model had good clinical utility in predicting PDAP.</p><p><strong>Conclusion: </strong>The proposed nomogram exhibited excellent predictive performance and clinical utility, providing a valuable tool for early identification and intervention in PDAP. Further external validation and prospective studies are recommended.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"248"},"PeriodicalIF":2.2,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12090575/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144101310","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Pathological perspective reveals a novel hemodialyzer reaction: a case report.","authors":"Weijuan Lou, Yongchun Xi, Ya Liu, Xueling Cai, Junfang Gai, Jianyong Yin, Jiahui Ding, Yifang Yang, Yanjuan Teng, Tingfang Chen, Niansong Wang, Yongping Guo","doi":"10.1186/s12882-025-04180-6","DOIUrl":"10.1186/s12882-025-04180-6","url":null,"abstract":"<p><strong>Background: </strong>While the appearance of red clots in the dialyzer and the arterial and venous blood tubing lines is a common phenomenon in every hemodialysis unit, the occurrence of recurrent yellowish-white matter formation in the hemodialysis venous blood pot of a patient is rare.</p><p><strong>Case presentation: </strong>We describe a male 69-year-old male with recurrent yellowish-white matter formation in the hemodialysis venous blood pot and red clots in the dialyzer. This was associated with a significant decrease in his red blood cells count. He had no history of thrombus no pro-thrombotic risk factors could be identified. Light microscopic examination of the deposits revealed the presence of large aggregates of neutrophils, large amounts of fibrin. The yellowish-white matter recurred at the next dialysis session. The occurrence of this episode was completely resolved by switching the dialysis filter and could not be avoided by increasing low molecular weight heparin dosage.</p><p><strong>Conclusion: </strong>The yellowish-white matter and clotting within the dialyzer, as well as severe anemia, could be prevented by changing the type of dialyzer. Due to the rarity of this dialyzer reaction, it is important that awareness of this reaction by early identification be undertaken.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"246"},"PeriodicalIF":2.2,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12090545/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144101313","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Gender-specific sarcopenia screening in hemodialysis: insights from lower limb strength and physiological indicators.","authors":"Yujie Yang, Hualong Liao, Yang Chen, Ying Qiu, Fei Yan, Ping Fu, Jirong Yue, Yu Chen, Huaihong Yuan","doi":"10.1186/s12882-025-04176-2","DOIUrl":"10.1186/s12882-025-04176-2","url":null,"abstract":"<p><strong>Objective: </strong>Maintenance hemodialysis (MHD) patients often suffer from sarcopenia, affecting lower limb muscle strength and increasing the risk of falls and mortality. This study aims to develop an auxiliary screening model for sarcopenia in MHD patients based on machine learning methods, utilizing lower limb muscle strength indicators, while paying attention to the gender difference and exploring its value in sarcopenia screening.</p><p><strong>Methods: </strong>This cross-sectional study collected data from MHD patients at a hemodialysis center in China. Sarcopenia was assessed using the 2019 Asian Working Group for Sarcopenia update. A self-developed lower limb muscle strength testing device was used. Other physiological indicators, including basic information and lab findings, were collected. Participants were grouped into sarcopenia and control groups, with gender-specific binary classification models developed. Stratified shuffling and synthetic minority oversampling techniques were used to build screening classifiers.</p><p><strong>Results: </strong>Data from 164 MHD patients were ultimately collected, including 83 males (41 with possible sarcopenia or sarcopenia) and 81 females (53 with possible sarcopenia or sarcopenia). Gender-specific binary classification models were developed using lower limb muscle strength indicators, with the male model having an AUC of 79% and the female model an AUC of 80%, respectively. Combining lower limb muscle strength with other physiological indicators improved the female model's screening capability, achieving an AUC of 90%.</p><p><strong>Conclusion: </strong>This study demonstrates that the auxiliary screening model for sarcopenia, developed using machine learning methods, highlights the significant value of lower limb muscle strength indicators in identifying sarcopenia in MHD patients. The gender-specific screening models show good discriminatory ability across different genders, providing effective tools for the early screening and management of sarcopenia in MHD patients.</p><p><strong>Trial registration: </strong>Chinese Clinical Trial Registry (ChiCTR2100051111), registered on 2021-09-13.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"247"},"PeriodicalIF":2.2,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12090688/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144101312","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BMC NephrologyPub Date : 2025-05-19DOI: 10.1186/s12882-025-04155-7
Sarah N Ali, Nicole Fusco, Dilip Makhija, Vakaramoko Diaby, Tosin Oladapo, Darsh Devani, Cibele Pinto, Mohit Mathur, Ancilla W Fernandes
{"title":"Burden of corticosteroid therapy in patients with immunoglobulin A nephropathy (IgAN): a systematic literature review.","authors":"Sarah N Ali, Nicole Fusco, Dilip Makhija, Vakaramoko Diaby, Tosin Oladapo, Darsh Devani, Cibele Pinto, Mohit Mathur, Ancilla W Fernandes","doi":"10.1186/s12882-025-04155-7","DOIUrl":"10.1186/s12882-025-04155-7","url":null,"abstract":"<p><strong>Background: </strong>Immunoglobulin A nephropathy (IgAN) is one of the most common forms of primary glomerulonephritis (GN) worldwide. While specific treatment differs regionally, treatment usually focuses on background therapy, with short-term (≤ 6 months) corticosteroids recommended as an add-on treatment for patients at high risk of progressive chronic kidney disease. Although corticosteroids can help to manage IgAN, treatment with corticosteroids may lead to undesirable adverse outcomes.</p><p><strong>Objective: </strong>To highlight corticosteroid treatment burden in patients with IgAN globally.</p><p><strong>Methods: </strong>Embase, MEDLINE, and Cochrane CENTRAL were searched for articles published in any language from January 1, 2013 to August 24, 2023. Eligible studies reported ≥ 1 outcome related to the clinical, humanistic, or economic burden of corticosteroids in patients with IgAN. Articles were independently screened by 2 reviewers. Data extraction and quality assessment were completed by 1 researcher and validated by a second. Results are reported among the number of studies with data on each outcome.</p><p><strong>Results: </strong>Of 1,024 records screened, 64 studies were included. Of 37 studies reporting treatment duration, 68% found that corticosteroids were used long-term (range: 8-24 months). In studies reporting data for long-term use (> 6 months), there were more overall AEs and serious AEs with corticosteroids than with comparator treatments (e.g., background therapy alone, tonsillectomy, placebo). Rates of metabolic AEs, Cushing's syndrome, edema and sleep disorders were also higher with long-term corticosteroids than with comparator treatments; however, most studies did not report the statistical significance of these results. Infection rates were similar between corticosteroids and comparator treatments.</p><p><strong>Conclusions: </strong>Current guidelines recommend short-term corticosteroid treatment for patients at high risk of progression but long-term use appears to be widespread. Corticosteroids may lead to adverse outcomes and should therefore be reserved only for IgAN patients most at risk of rapid progression to end-stage kidney disease and for limited duration. Novel corticosteroid-sparing therapies are necessary to supplement the current treatment landscape.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"249"},"PeriodicalIF":2.2,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12090507/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144101311","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BMC NephrologyPub Date : 2025-05-16DOI: 10.1186/s12882-025-04164-6
Ajay Kumar, Anirban Mazumdar, A K Bhattacharjee, Arvind Gupta, Arundhati Dasgupta, Binayak Sinha, Banshi Saboo, Chitra Selvan, Ghanshyam Goyal, Jaganmohan Balaji, Krishna G Seshadri, Kalyan K Gangopadhyay, G Vijay Kumar, Manoj Chawla, Mohua Sikdar, Nilakshi Deka, N K Singh, Purvi Chawla, Pratap Jetwani, Rajiv Kovil, Samit Ghosal, Subir Ray, Sudip Chatterjee, Sruti Chandrasekharan, Sambit Das, Subhajyoti Ghosh, Sonali Patange, Sanjay Reddy, T Surekha
{"title":"Risk factors associated with Indian type 2 diabetes patients with chronic kidney disease: CITE study, a cross-sectional, real-world, observational study.","authors":"Ajay Kumar, Anirban Mazumdar, A K Bhattacharjee, Arvind Gupta, Arundhati Dasgupta, Binayak Sinha, Banshi Saboo, Chitra Selvan, Ghanshyam Goyal, Jaganmohan Balaji, Krishna G Seshadri, Kalyan K Gangopadhyay, G Vijay Kumar, Manoj Chawla, Mohua Sikdar, Nilakshi Deka, N K Singh, Purvi Chawla, Pratap Jetwani, Rajiv Kovil, Samit Ghosal, Subir Ray, Sudip Chatterjee, Sruti Chandrasekharan, Sambit Das, Subhajyoti Ghosh, Sonali Patange, Sanjay Reddy, T Surekha","doi":"10.1186/s12882-025-04164-6","DOIUrl":"10.1186/s12882-025-04164-6","url":null,"abstract":"<p><strong>Background: </strong>Type 2 diabetes (T2DM) is the leading cause of chronic kidney disease (CKD) worldwide. Identifying clinical and laboratory associations with chronic kidney disease (CKD) in type 2 diabetes (T2DM) can help physicians target modifiable risk factors. In light of limited data from India, the CITE (CKD in Indian T2DM Evaluation) study was conducted.</p><p><strong>Methods: </strong>The multicenter, cross-sectional CITE study included 3,325 patients from 28 centres across India over a three-month period. CKD was defined as a persistent decline in kidney function (eGFR < 60 ml/min/1.73 m² for ≥ 3 months) or an elevated urine albumin-to-creatinine ratio (UACR) in at least two samples. Descriptive statistics summarised patient characteristics, while logistic regression analyses identified significant risk factors for CKD.</p><p><strong>Results: </strong>The prevalence of CKD in T2DM was 32%, with a median patient age of 59.9 years and 60.72% having a T2DM duration > 10 years. Reduced eGFR (< 60 ml/min/1.73 m²) was associated with older age (OR: 2.47, 95% CI 2.11-2.88, P < 0.001), longer T2DM duration (OR: 2.28, 95% CI 1.77-2.93, P < 0.001), higher HbA1c (OR: 1.039, 95% CI 1.001-1.079, P = 0.046), and elevated SBP (OR: 1.005, 95% CI 1.002-1.009, P = 0.003). Macroalbuminuria (UACR > 300 mg/g) was linked to non-vegetarian diet (OR: 1.95, 95% CI: 1.59-2.40, P < 0.001) and tobacco use (OR: 1.42, 95% CI: 1.17-1.73, P < 0.001). CKD increased comorbidity odds.</p><p><strong>Conclusion: </strong>The CITE study highlights the prevalence of CKD (32%) in Indian patients with T2DM and identifies clinical and laboratory factors associated with CKD, including age ≥ 60 years, T2DM duration, SBP, HbA1c, tobacco use, non-vegetarian diet, and comorbidities. Longitudinal studies are needed to confirm these associations and evaluate causality.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"245"},"PeriodicalIF":2.2,"publicationDate":"2025-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12083097/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144085846","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Prognostic value of glycemic gap in ST-segment elevation myocardial infarction-associated acute kidney injury.","authors":"Xiaofu Zhang, Yong Li, Qinghuan Yang, Siwen Wu, Yang Song, Ziyun Luo, Jianping Xu","doi":"10.1186/s12882-025-04167-3","DOIUrl":"10.1186/s12882-025-04167-3","url":null,"abstract":"<p><strong>Background: </strong>Stress-induced hyperglycemia (SIH) is a common phenomenon in acute myocardial infarction and is associated with poor prognosis. The relationship between glycemic gap (GG), a marker of SIH, and ST-segment elevation myocardial infarction (STEMI)-associated acute kidney injury (STAAKI) remains unclear. This study aims to explore the predictive value of GG for the risk of STAAKI after percutaneous coronary intervention (PCI) in STEMI patients.</p><p><strong>Methods: </strong>This study retrospectively selected patients diagnosed with STEMI who underwent primary PCI. Logistic regression analysis was used to identify the risk factors associated with STAAKI. To examine the dose-response relationship between GG and STAAKI, restricted cubic splines (RCS) were employed. The predictive accuracy of the models was assessed using Delong test, net reclassification index (NRI) and integrated discrimination improvement (IDI).</p><p><strong>Results: </strong>This study included 595 patients, the incidence of STAAKI was 9.2%. Multivariate logistic regression showed LVEF (OR per 1% increase = 0.931, 95% CI: 0.895 ~ 0.969), NT-proBNP (OR per 1 pg/mL increase = 1.579, 95% CI: 1.212 ~ 2.057), and GG (OR per 1 mmol/L increase = 1.379, 95% CI: 1.223 ~ 1.554) as independent predictors of STAAKI. RCS analysis indicated a linear dose-response relationship between GG and STAAKI. After integrating GG, the new model could significantly improve the risk model for STAAKI (Z = 2.77, NRI = 0.780, and IDI = 0.095; All P < 0.05).</p><p><strong>Conclusion: </strong>GG is an independent risk factor for the occurrence of STAAKI after PCI in STEMI patients, and integrating GG can significantly improve risk modeling regarding STAAKI.</p><p><strong>Clinical trial number: </strong>Not applicable.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"243"},"PeriodicalIF":2.2,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12080177/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144075868","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BMC NephrologyPub Date : 2025-05-15DOI: 10.1186/s12882-025-04166-4
Vladimir Petrovic, Andrew Whiteman, Matt Peach, Sam Kim, Vladislav A Malkov, Grant Budas, Andrew N Billin
{"title":"Plasma proteome signatures of ASK1 inhibition by selonsertib associate with efficacy in the MOSAIC randomized trial for diabetic kidney disease.","authors":"Vladimir Petrovic, Andrew Whiteman, Matt Peach, Sam Kim, Vladislav A Malkov, Grant Budas, Andrew N Billin","doi":"10.1186/s12882-025-04166-4","DOIUrl":"10.1186/s12882-025-04166-4","url":null,"abstract":"<p><p>Oxidative stress is a driver of acute and chronic kidney injury. Selonsertib is a clinical stage antagonist of ASK1 (MAP3K5), a serine/threonine kinase that is a mediator of oxidative stress signaling pathways. Selonsertib has demonstrated promising effects on preserving kidney function in the Phase2b Diabetic Kidney Disease (DKD) MOSAIC trial. However, little is known about the biological effects of ASK1 inhibition by selonsertib and its potential mechanism of action in DKD. We identified a plasma proteome signature of selonsertib activity that implicates numerous signaling pathways that regulate fibrosis, inflammation and oxidative stress response demonstrating translation of non-clinical models to the clinic. We further demonstrate that the effects of selonsertib on the plasma proteome are most pronounced in a subset of patients with poor baseline kidney function but who respond well to selonsertib treatment. This observation has implications for the future development of ASK1 inhibitors in a distinct patient population with DKD.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"244"},"PeriodicalIF":2.2,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12080038/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144075864","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BMC NephrologyPub Date : 2025-05-15DOI: 10.1186/s12882-025-04170-8
Wenji Wang, Lin Chen, Feng Ding, Xuezhu Li
{"title":"A case report of atypical autosomal dominant polycystic kidney disease presenting as glomerulocystic kidney superimposed with thin basement membrane nephropathy.","authors":"Wenji Wang, Lin Chen, Feng Ding, Xuezhu Li","doi":"10.1186/s12882-025-04170-8","DOIUrl":"10.1186/s12882-025-04170-8","url":null,"abstract":"<p><strong>Background: </strong>Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent monogenic polycystic kidney disorder, but ADPKD presented as Glomerulocystic kidney (GCK) in adults is uncommon. Thin basement membrane nephropathy (TBMN) seems to account for major causes of familial hematuria and can coexist with other glomerular diseases. Here, we report a case of atypical manifestation of ADPKD presenting as GCK superimposed with TBMN in an adult man.</p><p><strong>Case presentation: </strong>A 40-year-old male presented with moderate proteinuria, microhematuria and renal insufficiency. He has no family history of kidney disease. Ultrasound revealed slightly enlarged kidneys with echogenic cortex. 2 to 3 visible small cortical cysts on both kidneys, and no anatomical abnormalities were detected by CT scan. Renal biopsy demonstrated that 33.3% (9/27) of the glomeruli had marked dilatation of Bowman's space. The glomerular cysts were lined by a simple layer of cuboidal epithelium, which was stained positive for Claudin-1 (parietal epithelial cell marker), but negative for LTA and DBA (tubular epithelial cell markers). There were foci of mild chronic interstitial fibrosis with few inflammatory infiltrates. Immunofluorescence stains were negative. Transmission Electron microscopy (TEM) revealed extensive glomerular basement membrane (GBM) thinning, without splitting or lamellation. The average thickness of GBM was 221 ± 25 nm. No electron dense deposits were identified by TEM. The next-generation sequencing indicated pathogenic heterozygous deletion of PKD1 exon 3, and the mutation was determined to be a de novo mutation by familial variant analysis. No pathogenic mutations of COL4A3, COL4A4, COL4A5, UMOD, TCF2 and HNF1β were identified.</p><p><strong>Conclusion: </strong>We report a rare case of atypical ADPKD presenting as GCK superimposed with TBMN. GCK is a rare disease and often overlooked. It is important for practicing nephrologists to have a clear understanding of GCK. GCK involves in various conditions, thus genetic analysis should be considered.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"242"},"PeriodicalIF":2.2,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12080022/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144075839","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BMC NephrologyPub Date : 2025-05-15DOI: 10.1186/s12882-025-04156-6
Miguel G Uriol-Rivera, Frank R Ernst, John N Booth, Àngels Comas, Christoph Gasteyger, Ioannis Tomazos, Ching Lum, Yan Wang, Ana I Ávila
{"title":"PLASMIC score to aid diagnosis of aHUS: an analysis of C5 inhibitor clinical trials and the PINC AI™ healthcare database.","authors":"Miguel G Uriol-Rivera, Frank R Ernst, John N Booth, Àngels Comas, Christoph Gasteyger, Ioannis Tomazos, Ching Lum, Yan Wang, Ana I Ávila","doi":"10.1186/s12882-025-04156-6","DOIUrl":"10.1186/s12882-025-04156-6","url":null,"abstract":"<p><strong>Background: </strong>Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) that can lead to end organ damage and death without treatment. The ability to rapidly distinguish aHUS from other forms of TMA is key for optimal patient management. The PLASMIC Score was developed to identify individuals with thrombotic thrombocytopenic purpura (TTP), a TMA subtype characterized by severe ADAMTS13 deficiency (< 10%), using 7 commonly available laboratory variables and aspects of the patient's medical history. This study aimed to assess the distribution of PLASMIC Scores in patients with known aHUS, and evaluate the utility of the PLASMIC Score in the diagnostic pathway of aHUS in patients with confirmed TMA.</p><p><strong>Methods: </strong>Data from eculizumab (NCT01194973) and ravulizumab (NCT02949128) clinical trials were utilized to calculate and evaluate PLASMIC Score distribution in aHUS patients. Real-world patient-level data from the PINC AI™ Healthcare Database (PHD) were used to evaluate the performance of the PLASMIC Score in identifying aHUS in patients with documented TMA diagnoses and renal impairment (primary analysis population; n = 110), and subsequent sensitivity analyses were performed in alternative populations.</p><p><strong>Results: </strong>A total of 94 aHUS patients from the eculizumab and ravulizumab clinical trials dataset were evaluated; 18/36 (50.0%) and 27/58 (46.6%) patients in the eculizumab and ravulizumab trials, respectively, had a PLASMIC Score of 4, and most patients (~ 85%) had PLASMIC Scores ≤ 5 (range: 3-5), which were distributed similarly between the trials. Among the 110 patients with undifferentiated TMA (primary analysis) from the PHD, a PLASMIC Score cutoff of ≤ 5 yielded sensitivity, specificity and positive predictive value (PPV) and negative predictive values (NPV) of 86.5%, 71.4%, 92.8% and 55.6%, respectively, for identifying probable aHUS. Similar diagnostic performance was observed at a cutoff value of ≤ 5 in further sensitivity analyses. A cutoff value of ≤ 4 yielded a lower PPV (62.9%), yet a higher NPV (85.7%), with only 3 patients misclassified as TTP.</p><p><strong>Conclusion: </strong>Application of the PLASMIC Score in the aHUS diagnostic pathway may support clinical judgement and ascertain confidence in the earlier identification and subsequent treatment of patients with aHUS, thereby improving patient outcomes.</p>","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"241"},"PeriodicalIF":2.2,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12080142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144075866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BMC NephrologyPub Date : 2025-05-14DOI: 10.1186/s12882-025-04152-w
Linda H Ficociello, Rachel Lasky, Hans-Juergen Arens, Despina Ruessmann, Michael S Anger
{"title":"Correction: Real-world use of difelikefalin in hemodialysis patients at a large dialysis organization in the United States: a retrospective database study.","authors":"Linda H Ficociello, Rachel Lasky, Hans-Juergen Arens, Despina Ruessmann, Michael S Anger","doi":"10.1186/s12882-025-04152-w","DOIUrl":"https://doi.org/10.1186/s12882-025-04152-w","url":null,"abstract":"","PeriodicalId":9089,"journal":{"name":"BMC Nephrology","volume":"26 1","pages":"240"},"PeriodicalIF":2.2,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12080169/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144075853","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}